Summary: The common variable immunodeficiency (IDCV) comprises a heterogeneous group of primary immune defects that are characterized by repeated infections and hipogammaglobulinèmia. This is the primary immunodeficiency (IDPs) more frequent after the deficit IgA (DIgA) and debuted at any age, mostly in adulthood. The purpose of this work have been: define the clinical and immunological parameters of the IDCV in the pediatric age and the difficulty in diagnosis, differential define the parameters regarding the DIgA, assess treatment outcome, the aftermath i mortality of IDCV in i study the pediatric age family history of cases of IDCV and DIgA in the pediatric age. It has conducted a retrospective study of cases diagnosed IDCV and DIgA symptomatic at the Children's Hospital Vall d'Hebron from
1980 to 2003, with 19 cases IDCV and 30 cases of DIgA symptomatic. It has gathered an extensive database for both the diagnosis and monitoring. The mean age at diagnosis IDCV was 8 years old, with a predominance of males (68%). The median follow-up was 12 years. The most frequent clinical manifestations were infections repetition (89% to diagnosis) dominance of respiratory, allergy (68%), b ronquiectasias (32%), autoimmunity (21%), delayed pondo - estatural (21%) and vasculitits (5%, 1 single case). There are no cases of malignancies or exitus. Regarding laboratory findings in cases of IDCV: the amount of IgG in the diagnosis averaged 290 mg / dl and shows no relation to the severity or prognosis, even with age, but it shows correlation with the presence of bronchiectasis. The number of NK at diagnosis was also significantly higher in cases with bronchiectasis in the diagnosis, monitoring disappearing. The number of B lymphocytes (LB) low relates to the most serious cases and poor prognosis, especially when we refer to the LB memory (CD19 + CD27 + IgD-). Among the family background of the cases with IDCV stresses that a 2 6% of children have other IDCV in the family. Cases of DIgA symptomatic presented clinical manifestations similar peromenos severe and frequent but also predominance of infections repetition and increase in allergies and autoimmune processes, but not detect any bronchiectasis. Among the family background, 30% of children with DIgA symptomatic family had some other IDP (most DIgA) and a 13% IDCV. CONCLUSIONS * IDCV in the pediatric age presents a predominance of male and the onset of symptoms is more early in children over girls. * The sex femeninio are more common autoimmune manifestations. * The administration of intravenous gammaglobulin prevent new infections, neutralizes existing and improves lung inflammatory response. * The delay in diagnosis increases the proportion of bronchiectasis. Therefore, early diagnosis is essential and improves the quality of life for these children. * The number of low LB relates to the severity of clinical manifestations, especially when accompanied by LB memory deficits. * The number of NK high relates to the existence of bronchiectasis. No significant differences in the number and function of T cells * The number of children with IDCV family is high: 26% in our series. * The type of infections of DIGa symptomatic infections are similar to the cases with IDCV, but less severe and frequent. * The number of cases relatives of IDCV i DIgA and cases of DiGa symptomatic progressing to IDCV make regular monitoring of these children and their families is very important. It requires periodic evaluations.
